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During the congress, E-Posters will be accessible to all participants on the congress website 24/7, as well as in the E-poster stations in the congress center.
Preparing your E-Poster
Please review the E-Poster format requirements carefully when preparing your E-Poster. Should your E-Poster not meet the mentioned requirements, it may not be displayed as described above.
E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
Please follow the instructions below to input your abstract title.
Abstract titles should be brief and reflect the content of the abstract.
Recessive dystrophic epidermolysis bullosa (RDEB) is hereditary skin disorder, leading recurrent skin blisters, caused by COL7A1 gene mutation coding collagen type VII. Kidney complications in patients with epidermolysis bullosa have been reported including IgA nephropathy, infection-related glomerulonephritis (IRGN), C3 glomerulonephritis and AA amyloidosis. However, a pathogenesis of these kidney injuries remains unclear.
Case presentations
<Case1>
A woman in her 40s was diagnosed RDEB in adulthood. She underwent kidney biopsy due to rapid progressive glomerulonephritis (RPGN)-like kidney injury. Laboratory findings showed serum creatinine (Cr) 1.86mg/dL, urinary-red blood cells (U-RBC)>100/HPF, urinary protein (UP) 3.4 g/gCr, CRP 5.9 mg/dL, IgG 4877 mg/dL, IgA 659 mg/dL, IgM 47 mg/dL. Staphylococcus aureus had been previously isolated from a skin lesion. Pathological findings revealed cellular or fibrocellular crescents in 9 of 51 glomeruli, mesangial IgA (1+) and C3 (2+) deposition on immunofluorescence (IF) and subepithelial hump-like electron dense deposit (EDD) in electron microscopy (EM).
<Case2>
A woman in her 50s was diagnosed RDEB at birth. Microscopic hematuria had developed 8 years earlier and she underwent kidney biopsy for worsening kidney function and proteinuria. Laboratory findings showed Cr 1.63 mg/dL, U-RBC>100/HPF, UP 0.9 g/gCr, CRP 8.4 mg/dL, IgG 4633mg/dL, IgA 1024 mg/dL, IgM 67 mg/dL. Staphylococcus aureus had been previously isolated from a skin lesion. Pathological findings revealed cellular crescent in 1 of 67 glomeruli, mesangial IgA (±), C3 (1+) deposition on IF and subepithelial hump-like EDD in EM.
Both patients presented chronic inflammation and had a history of Staphylococcus aureus isolation from skin cultures. Kidney biopsies demonstrated focal crescentic glomerulonephritis with mainly C3 deposition and hump-like subepithelial deposit, suggesting IRGN features. Previous reported cases also showed mainly C3 positive glomerulonephritis with various EDDs including subepithelial deposit, which were originally diagnosed IgA nephropathy or C3 glomerulonephritis in the literatures.
Considering these two cases and previous reports, we assumed that the etiology of glomerulonephritis is IRGN triggered by skin infection resulting from skin barrier disruption, representing a possible form of epidermolysis bullosa associated nephropathy.
The content presented in this abstract was also submitted for the 55th Eastern Regional Meeting of the Japanese Society of Nephrology.
